What is pulmonary fibrosis?

Pulmonary fibrosis (lung scarring) describes a group of interstitial lung diseases where the lungs become progressively stiffer and smaller, eventually leading to low oxygen levels in the blood. Pulmonary fibrosis (PF) affects around 70,000 people in the UK.

Symptoms of pulmonary fibrosis

Scar tissue or fibrosis in the lungs impairs how well the alveoli can transfer oxygen that is breathed in, eventually leading to low oxygen levels in the blood. As the amount of scar tissue increases, the lungs become smaller.

The symptoms of pulmonary fibrosis may include:

  • shortness of breath, which may occur with exercise initially but it worsens over time
  • a persistent cough
  • rounded and swollen finger tips (finger clubbing)
  • crackles over your lungs heard by your doctor using a stethoscope

Shortness of breath

Breathlessness can be an early or late PF symptom.  People often write off shortness of breath, putting it down to lack of fitness and ageing.

In the early stages of PF, you may be breathless when you do strenuous exercise or walk up hills or stairs. As the disease progresses, you may notice breathlessness with milder activities such as walking, washing, dressing, talking and eating.

Read our advice on managing breathlessness and pulmonary rehabilitation.

Cough and crackles over the lungs

A persistent dry and tickly cough is a common symptom of pulmonary fibrosis. Sometimes the cough can be productive of sticky sputum. Often patients visit their GP with a cough. On listening to the lungs, the GP hears dry or “Velcro”-type crackles that are a strong indicator of PF.

Read our advice on managing a cough.

Rounded and swollen finger tips

Finger clubbing – the widening and rounding of the fingernails or toenails – is a less common pulmonary fibrosis symptom. At the moment we don’t know why some patients develop this and others don’t.

Other symptoms

Other pulmonary fibrosis symptoms may include tiredness or unintentional weight loss.

Some people develop pulmonary hypertension, where there is increased blood pressure within the arteries of the lungs. You may have chest pains, a racing heartbeat and swelling of your ankles or legs. Your medical team is likely to carry out tests for pulmonary hypertension.

If the pulmonary fibrosis is related to an autoimmune disease, such as rheumatoid arthritis or scleroderma (systemic sclerosis), you may also experience symptoms of that condition  – for example, problems with joints, skin, or muscles.

Causes of pulmonary fibrosis

Pulmonary fibrosis occurs when collagen (scar tissue, often known as ‘fibrosis’) accumulates within the delicate gas-exchanging air sacs (alveoli) of the lungs. As a result, the lungs become stiffer and smaller. This eventually results in low oxygen levels in the blood.

Pulmonary fibrosis conditions are classified by their causes.

Doctors understand the causes of hypersensitivity pneumonitis, asbestosis and pulmonary fibrosis caused by autoimmune diseases, such as rheumatoid arthritis and systemic sclerosis (scleroderma). Occasionally, some medications, can cause drug-induced pulmonary fibrosis.  But it is not yet clear why some people develop these conditions, while others don't.

There is currently no known cause of idiopathic pulmonary fibrosis.

Pulmonary fibrosis diagnosis

Before making a diagnosis of lung fibrosis, your medical team will ask you questions about your breathing problems, examine you and arrange specific tests.

The tests and the clinical information taken together are reviewed in a multi-disciplinary team meeting to help the medical team understand your pulmonary fibrosis condition and recommend the most appropriate treatment.

Questions about your breathing

  • Your symptoms, including coughing and breathlessness
  • Jobs you have had, particularly where you may have been exposed to asbestos, dust from wood or stone, metals, birds or animals
  • Your hobbies and pets
  • Smoking
  • Family history of lung disease
  • Your general health

Tests to diagnose pulmonary fibrosis

Pulmonary fibrosis diagnosis involves a number of tests:

  • Blood tests to assess for autoimmune diseases
  • In a lung function test, you will breathe  into a tube so that your medical team can see how well your lungs work. The  tests will identify your lung capacity and how well your lungs transfer oxygen to and from your blood. This will help to inform your treatment plan.
  • 6 minute walking test to assess if your blood oxygen level falls when you walk.
  • chest x-ray can indicate whether you have scarring in your lungs. Pulmonary fibrosis often starts in the lower part of your lungs whereas sarcoidosis usually involves the mid and upper zones of the lungs.
  • A high-resolution CT scan will give a more detailed picture of the inside of your lungs. Typical features of pulmonary fibrosis include fine lines (called reticulations), ‘ground glass’ opacities and honeycombing patterns.
  • An echocardiogram looks at how well your heart is working. Increased pressure on the right side of the heart is a sign of pulmonary hypertension – which is a complication of pulmonary fibrosis.
  • bronchoscopy involves a small camera that looks at the airways inside your lungs and takes samples from the lung tissues (bronchial lavage). The camera is passed either through the nose or mouth into your lungs. You will have a local anaesthetic and will not usually have to spend the night at hospital.
  • You may also have a bronchial lavage as part of the bronchoscopy, in which small amounts of salty water are passed into the lungs and sucked back out, sampling cells from the lungs that can be analysed to inform your diagnosis.
  • surgical lung biopsy is sometimes necessary when other tests haven’t provided enough information for a certain diagnosis. This is usually performed by keyhole approach called video-assisted thoracoscopic surgery(VATS). You will have a general anaesthetic and have to spend a  few days in hospital.

Treatments for pulmonary fibrosis

There are 23 specialist Interstitial Lung Disease (ILD) centres in the UK, with doctors, nurses, and allied health professionals dedicated to the treatment of pulmonary fibrosis.

Treatment depends on the types and causes of pulmonary fibrosis. There are drug treatments for IPF and treatments for other types of PF. New treatments are being developed through clinical trials all the time.

There are a number of treatments to help with symptom control.  These include:

  1. breathlessness management and pulmonary rehabilitation
  2. oxygen therapy – if blood oxygen levels fall with exercise or when resting  
  3. cough suppressants and mucolytics to aid sputum clearance
  4. management of anxiety and panic attacks

Your doctor may suggest assessment by palliative and supportive care specialists for helping with symptom control and psychological support.

If you smoke, there is help to stop smoking.

For some people, referral for lung transplantation may be discussed.  

There are a number of novel therapies being evaluated in clinical trials for IPF.  Please discuss with your doctor if you are interested in finding out about clinical trials.

Pulmonary fibrosis conditions:

Pulmonary fibrosis outlook and prognosis

Progressive pulmonary fibrosis is a life-limiting disease. While trends in life expectancy are improving, no one can tell an individual exactly how long they will live. Research that suggests an average life expectancy of between 3–5 years, was carried out before treatments that can slow down the rate of lung scarring were widely available. There are many factors that will affect life expectancy such as the cause of pulmonary fibrosis, a person’s age and any underlying health conditions.

What to expect from your care

After your diagnosis, you’ll have regular follow-up appointments with your medical team. They will explore your symptoms and discuss with you whether your treatment needs to change.

Get support

Join a support group in your area

It’s important to remember that you are not alone. There are now over 75 pulmonary fibrosis support groups around the country.

Support groups meet informally to share experiences, provide expert information and raise funds and awareness. Find a group near you.

Call our support line

APF runs an email and telephone support line offering information and advice about living with PF. Find out more.

Find us on social media

We have an active and supportive online community ready to welcome you on Facebook, Twitter and Instagram for updates, news and connections to our online community.

Watch videos of how other people cope day to day with PF.

The work APF is doing for individuals and families affected by PF is great and I would like to help in any way I can.

For healthcare professionals

Information provided by Action for Pulmonary Fibrosis (APF) is not a substitute for professional medical advice. It’s intended as general information only. APF is not responsible for any errors or omissions or for any loss or damage suffered by users resulting from the information published on actionpf.org.

Further information for carers:

Your essential caring guide (PDF)
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